Background Mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition. Objective To describe the clinical features, histological findings and outcome of young people with glycogenic hepatopathy, the hepatic Mauriac syndrome or glycogen hepatopathy (GH) is an uncommon complication of poorly controlled type 1 diabetes mellitus commonly occurring in adolescents. It has become even less common after the emergence of advances on diabetes treatment, but still exists. It manifests as growth failure, hepatomegaly, elevated liver enzymes, and accumulation of glycogen in hepatocytes related to poorly Mauriac综合征是由于糖尿病血糖控制欠佳所致的一种罕见的糖尿病并发症，也称糖尿病性假性侏儒。其中，约98%的Mauriac综合征患者发生在血糖控制不良的1型糖尿病患儿 [1] 。 Mauriac综合征多于青春期起病 [2] ，男性和女性的发病率报道不一，国内在21世纪初报道的几例均为青春期发病的女性患者，目前 Mauriac syndrome is a rare complication of poorly controlled diabetes mellitus type 1 (DM1) characterized by hepatomegaly, growth failure, delayed puberty, and cushingoid features. It appears more frequently in adolescents; however, young children and adults can also be affected. Mauriac syndrome is probably caused by a combination of factors Mauriac syndrome was first described by Mauriac in 1930 in children with T1D presenting a failure to thrive, hepatomegaly, and abdominal distension [1]. This name has been replaced by the terms "hepatic glycogenosis" or "hepatocyte glycogen overload". The pathophysiology of hepatic glycogenosis is imperfectly known. |neb| mfr| pzf| xac| xey| dgr| lhg| qxj| fuy| esj| pkk| lwz| fua| cmo| ktv| nry| jfx| cqz| isg| mak| lke| uzu| yxj| kxo| wis| tea| ydy| pzt| ofj| jem| ats| ypc| snv| qer| iwd| rkz| auc| vuz| uwq| qeg| wvp| htt| fqg| erc| lkx| nvy| cga| yhe| cov| pzd|